What is Shy-Drager syndrome?

Shy-Drager syndrome: A progressive disorder of the central and sympathetic nervous systems, also called multiple system atrophy with postural hypotension (an excessive drop in blood pressure when the patient stands up, causing dizziness or momentary blackouts).

What is Shy-Drager syndrome named after?

Shy-Drager syndrome is a rare, progressively degenerative disease of the autonomic nervous system. It is named after Dr Milton Shy and Dr Glenn Drager, who identified this syndrome in 1960.

Is Shy-Drager syndrome fatal?

Doctors base the diagnosis on a person’s response to levodopa (used to treat Parkinson disease) and results of magnetic resonance imaging and tests of autonomic function. Simple measures and drugs can help lessen symptoms, but the disorder is progressive and ultimately fatal.

Is Shy-Drager syndrome Rare?

Some of these features are similar to those seen in Parkinson’s disease, and early in the disease course it often may be difficult to distinguish these disorders. MSA is a rare disease, affecting potentially 15,000 to 50,000 Americans, including men and women and all racial groups.

What neurological causes orthostatic hypotension?

Among the primary causes of nOH are a group of neurodegenerative diseases characterized pathologically by the deposition of the protein α-synuclein in the central or peripheral nervous system, including Parkinson’s Disease (PD), MSA, Pure Autonomic Failure (PAF), and Dementia with Lewy Bodies (DLB).

Is MSA always fatal?

Multiple system atrophy (MSA) is a rare, sporadic, and ultimately fatal α-synucleinopathy with features of parkinsonism or cerebellar dysfunction, autonomic failure, and pyramidal dysfunction occurring in various combinations [1, 2].

What is the cause of Shy Drager Syndrome?

Today, Shy-Drager Syndrome (now known as called Multiple System Atrophy) is a neurological disease resulting from degeneration of certain nerve cells in the brain and spinal cord. Body functions controlled by these areas of the brain and spinal cord function abnormally in patients with this disease.

How do you test for neurogenic orthostatic hypotension?

Diagnosis of Neurogenic Orthostatic Hypotension The diagnosis of OH requires BP readings while supine and upright, either during active standing or during a tilt‐table test, to determine the presence of a sustained orthostatic fall of at least 20 mm Hg systolic or 10 mm Hg diastolic BP.

How did Shy Drager syndrome get its name?

Shy-Drager syndrome leads to dizziness or fainting when standing up, urinary incontinence, impotence, and muscle tremors. SDS was named for neurologists Milton Shy, M.D., from the National Institutes of Health, and Glenn Drager, M.D., from the Baylor College of Medicine, who first described the condition in 1960.

How long do people with Shy Drager syndrome live?

Shy Drager syndrome worsens with time and eventually leads to death and affected individuals survive an average of 10 years after the signs and symptoms first appear. However, the survival rate with Shy Drager syndrome varies widely.

How is orthostatic hypotension defined in Shy Drager syndrome?

Severe orthostatic hypotension is defined as a reduction in systolic blood pressure of at least 30mm Hg or in diastolic blood pressure of at least 15mm Hg, within 3 minutes of standing from a previous 3-minute interval in the recumbent position. This form of hypotension is common in Shy Drager syndrome, being present in at least 68% of patients.